Zhou Xiang-Xue, Qin Hao-Lin, Li Xun-Hua, Huang Hai-Wei, Liang Ying-Ying, Liang Xiu-Ling, Pu Xiao-Yong
Department of Neurology, The East Area of the First Affiliated Hospital, Guangzhou, China.
Neurol India. 2014 Jul-Aug;62(4):362-6. doi: 10.4103/0028-3886.141221.
The aim of this study was to evaluate the feasibility of characterizing the brain-mineral deposition in patients with Wilson disease (WD) using susceptibility-weighted imaging (SWI).
The study enrolled 30 WD patients and 20 age-matched healthy controls. Neurological symptoms were scored using the modified Young Scale. The hepatic function indices, serum and urinary copper content, and serum iron content were determined. All study objects received the magnetic resonance imaging (MRI) and SWI test of the brain. The values of corrected phase (CP) were calculated on SWI. The relationship between CP values and the clinical status were evaluated.
The serum iron content of WD patients was higher than the normal. The CP values of substantia nigra, caudate nucleus, and globus pallidus of WD were lower than the normal values, while the CP value of substantia nigra was the lowest. No correlations were determined between the CP values and the iron and copper parameters. There was negative correlation between the scores of dysarthria and the CP values of the globus pallidus. There was negative correlation between the scores of tremor and the CP values of caudate nucleus. Some regions, which had high signals on T2-weighted image, had low signals on SWI.
There might be abnormal iron metabolism in patients with WD. The decreased CP values might reflect a deposition of both copper and iron. SWI may be more sensitive than the ordinary MRI. The mineral deposition may contribute to the neural symptoms.
本研究旨在评估利用磁敏感加权成像（SWI）对威尔逊病（WD）患者脑矿物质沉积进行特征描述的可行性。
本研究纳入30例WD患者和20例年龄匹配的健康对照者。采用改良杨氏量表对神经症状进行评分。测定肝功能指标、血清和尿铜含量以及血清铁含量。所有研究对象均接受脑部磁共振成像（MRI）和SWI检查。在SWI上计算校正相位（CP）值。评估CP值与临床状况之间的关系。
WD患者血清铁含量高于正常水平。WD患者黑质、尾状核和苍白球的CP值低于正常值，其中黑质的CP值最低。CP值与铁和铜参数之间未发现相关性。构音障碍评分与苍白球CP值之间呈负相关。震颤评分与尾状核CP值之间呈负相关。一些在T2加权图像上呈高信号的区域，在SWI上呈低信号。
WD患者可能存在铁代谢异常。CP值降低可能反映了铜和铁的沉积。SWI可能比普通MRI更敏感。矿物质沉积可能导致神经症状。
